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Clinical characteristics and outcomes of primary intracranial alveolar soft-part sarcoma: A case report

BACKGROUND: Primary intracranial alveolar soft-part sarcoma (PIASPS) is a rare malignancy. We aimed to investigate the clinical profiles and outcomes for PIASPS. CASE SUMMARY: We firstly reported five consecutive cases from our institute. Then, the cases from previous studies were pooled and analyze...

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Autores principales: Chen, Jun-Yu, Cen, Bo, Hu, Fei, Qiu, Yong, Xiao, Guo-Min, Zhou, Jun-Ge, Zhang, Fang-Cheng
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Baishideng Publishing Group Inc 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8727262/
https://www.ncbi.nlm.nih.gov/pubmed/35071531
http://dx.doi.org/10.12998/wjcc.v10.i1.296
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author Chen, Jun-Yu
Cen, Bo
Hu, Fei
Qiu, Yong
Xiao, Guo-Min
Zhou, Jun-Ge
Zhang, Fang-Cheng
author_facet Chen, Jun-Yu
Cen, Bo
Hu, Fei
Qiu, Yong
Xiao, Guo-Min
Zhou, Jun-Ge
Zhang, Fang-Cheng
author_sort Chen, Jun-Yu
collection PubMed
description BACKGROUND: Primary intracranial alveolar soft-part sarcoma (PIASPS) is a rare malignancy. We aimed to investigate the clinical profiles and outcomes for PIASPS. CASE SUMMARY: We firstly reported five consecutive cases from our institute. Then, the cases from previous studies were pooled and analyzed to delineate the characteristics of this disease. Our cohort included two males and three females. The median age was 21-years-old (range: 8-54-years-old). All the patients received surgical treatment. Gross total resection (GTR), radiotherapy, and chemotherapy were administered in 3 patients, 4 patients, and 1 patient, respectively. After a median follow-up of 36 mo, tumor progression was noticed in 4 patients; and 3 patients died of the disease. Pooled data (n = 14) contained 5 males and 9 females with a median age of 19 years. The log-rank tests showed that GTR (P = 0.011) could prolong progression-free survival, and radiotherapy (P < 0.001) resulted in longer overall survival. CONCLUSION: Patients with PIASPS suffer from poor outcomes. Surgical treatment is the first choice, and GTR should be achieved when the tumor is feasible. Patients with PIASPS benefit from radiotherapy, which should be considered as a part of treatment therapies.
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spelling pubmed-87272622022-01-21 Clinical characteristics and outcomes of primary intracranial alveolar soft-part sarcoma: A case report Chen, Jun-Yu Cen, Bo Hu, Fei Qiu, Yong Xiao, Guo-Min Zhou, Jun-Ge Zhang, Fang-Cheng World J Clin Cases Case Report BACKGROUND: Primary intracranial alveolar soft-part sarcoma (PIASPS) is a rare malignancy. We aimed to investigate the clinical profiles and outcomes for PIASPS. CASE SUMMARY: We firstly reported five consecutive cases from our institute. Then, the cases from previous studies were pooled and analyzed to delineate the characteristics of this disease. Our cohort included two males and three females. The median age was 21-years-old (range: 8-54-years-old). All the patients received surgical treatment. Gross total resection (GTR), radiotherapy, and chemotherapy were administered in 3 patients, 4 patients, and 1 patient, respectively. After a median follow-up of 36 mo, tumor progression was noticed in 4 patients; and 3 patients died of the disease. Pooled data (n = 14) contained 5 males and 9 females with a median age of 19 years. The log-rank tests showed that GTR (P = 0.011) could prolong progression-free survival, and radiotherapy (P < 0.001) resulted in longer overall survival. CONCLUSION: Patients with PIASPS suffer from poor outcomes. Surgical treatment is the first choice, and GTR should be achieved when the tumor is feasible. Patients with PIASPS benefit from radiotherapy, which should be considered as a part of treatment therapies. Baishideng Publishing Group Inc 2022-01-07 2022-01-07 /pmc/articles/PMC8727262/ /pubmed/35071531 http://dx.doi.org/10.12998/wjcc.v10.i1.296 Text en ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved. https://creativecommons.org/licenses/by-nc/4.0/This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
spellingShingle Case Report
Chen, Jun-Yu
Cen, Bo
Hu, Fei
Qiu, Yong
Xiao, Guo-Min
Zhou, Jun-Ge
Zhang, Fang-Cheng
Clinical characteristics and outcomes of primary intracranial alveolar soft-part sarcoma: A case report
title Clinical characteristics and outcomes of primary intracranial alveolar soft-part sarcoma: A case report
title_full Clinical characteristics and outcomes of primary intracranial alveolar soft-part sarcoma: A case report
title_fullStr Clinical characteristics and outcomes of primary intracranial alveolar soft-part sarcoma: A case report
title_full_unstemmed Clinical characteristics and outcomes of primary intracranial alveolar soft-part sarcoma: A case report
title_short Clinical characteristics and outcomes of primary intracranial alveolar soft-part sarcoma: A case report
title_sort clinical characteristics and outcomes of primary intracranial alveolar soft-part sarcoma: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8727262/
https://www.ncbi.nlm.nih.gov/pubmed/35071531
http://dx.doi.org/10.12998/wjcc.v10.i1.296
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